Arrhythmogenic Channelopathy Syndromes Presenting as Refractory Epilepsy

Abstract Background Children and young adults with potentially lethal cardiac channelopathies often present to medical care with a history of syncope or seizures due to episodic ventricular arrhythmias and associated cerebral hypoperfusion. Methods Two important types of genetic arrhythmia syndromes...

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Bibliographic Details
Published in:Pediatric neurology 2013-08, Vol.49 (2), p.134-137
Main Authors: Hazle, Matthew A., MD, Shellhaas, Renée A., MD, MS, Bradley, David J., MD, Dick, Macdonald, MD, LaPage, Martin J., MD
Format: Article
Language:English
Subjects:
Adolescent
Channelopathies - complications
Channelopathies - diagnosis
Child
Electrocardiography
Electroencephalography
Epilepsy - diagnosis
Epilepsy - physiopathology
Female
Humans
Long QT Syndrome - complications
Neurology
Pediatrics
Tachycardia, Ventricular - complications
Tachycardia, Ventricular - diagnosis
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Summary:Abstract Background Children and young adults with potentially lethal cardiac channelopathies often present to medical care with a history of syncope or seizures due to episodic ventricular arrhythmias and associated cerebral hypoperfusion. Methods Two important types of genetic arrhythmia syndromes—long QT syndrome and catecholaminergic polymorphic ventricular tachycardia—are discussed using relevant case examples. The pathophysiology and distinguishing clinical features of these conditions are reviewed. Results The patients in each case were ultimately diagnosed with a cardiac channelopathy as the cause for their syncope and refractory seizures. With appropriate medical management, no further events have occurred to date. Conclusions Cardiac channelopathies can be misdiagnosed as refractory epilepsy when in fact these events represent convulsive syncopes. Knowledge of and suspicion for these arrhythmogenic conditions may expedite diagnosis and improve outcomes.
ISSN:0887-8994
1873-5150
DOI:10.1016/j.pediatrneurol.2013.03.017